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Effect of mexiletine on transitory depression of compound motor action potential in recessive myotonia congenita.

AbstractOBJECTIVE:
We aim to demonstrate the effect of mexiletine on the compound muscle action potential (CMAP) amplitude transitory depression (TD) in a cohort of patients with recessive myotonia congenita.
METHODS:
We evaluated 21 patients with recessive myotonia congenita referred to our institute from 1990 to 2013 and treated with mexiletine chlorhydrate. All patients underwent prolonged 3 Hz repetitive nerve stimulation (3 Hz-PLRS) before and after the beginning of treatment.
RESULTS:
We observed in all subjects a reduction of CMAP amplitude TD after the beginning of treatment. The mean value of the TD nadir before starting mexiletine treatment was -62.0% and reduced to -28.8% after the therapy was started (51.6% reduction, p<0.001).
CONCLUSIONS:
The 3 Hz-PLRS is configured as a neurophysiological test able to indirectly detect and quantify, through the measurement of TD, the clinical phenomenon of the transitory weakness that occurs in myotonic syndromes due to CLCN1 mutations.
SIGNIFICANCE:
This neurophysiological test might be considered a helpful tool to assess the effect of anti-myotonic drugs, as mexiletine, in recessive myotonia congenita.
AuthorsMauro Lo Monaco, Adele D'Amico, Marco Luigetti, Jean-François Desaphy, Anna Modoni
JournalClinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology (Clin Neurophysiol) Vol. 126 Issue 2 Pg. 399-403 (Feb 2015) ISSN: 1872-8952 [Electronic] Netherlands
PMID25065301 (Publication Type: Journal Article)
CopyrightCopyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
Chemical References
  • Voltage-Gated Sodium Channel Blockers
  • Mexiletine
Topics
  • Action Potentials (drug effects, physiology)
  • Adolescent
  • Adult
  • Aged
  • Child
  • Evoked Potentials, Motor (drug effects, physiology)
  • Female
  • Humans
  • Male
  • Mexiletine (pharmacology, therapeutic use)
  • Middle Aged
  • Myotonia Congenita (diagnosis, drug therapy, physiopathology)
  • Time Factors
  • Treatment Outcome
  • Voltage-Gated Sodium Channel Blockers (pharmacology, therapeutic use)
  • Young Adult

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